The incidence of cholangiocarcinoma will slowly decrease over time due to improvements in diagnostic technologies as well as an improved understanding of the biology of the development of sporadic and hereditary forms of this illness.
It is important to differentiate the types of cholangiocarcinoma, as it can affect many types of treatment strategies. In particular, a complete resection is more effective for large hilar CCCs than smaller choledochal or perihilar CCCs. The pTNM staging system is important in predicting tumour grade, which affects treatment decisions including surgical options.
Most cases of cholangiocarcinoma in India and USA is caused by infection with the liver flukes, Schistosoma haematobium and Opisthorchis viverrini. This is supported by the fact that most patients have bile duct pathology similar to that seen in schistosomiasis.
Treatment is largely directed toward the main tumor by surgical staging, surgical resection, or combinations of surgical and non-surgical therapies. Targeted therapies are now standard of care for cCRC.
Cholangiocarcinoma is an aggressive neoplasm of bile duct cells that frequently metastasize to the liver, regional lymph nodes or peritoneum. Even if the cancer is resected surgically, it may recur locally or metastasize. Patients with advanced, inoperable disease have a relatively poor survival. The treatment options for cholangiocarcinoma are limited, but the advent of improved surgical techniques and the development of innovative treatments may permit radical resection of primary tumors in the future.
Cholangiocarcinoma may present with jaundice or bile duct obstruction. Hepatocellular carcinoma presents with malaise and weight loss. Peritoneal metastasis may present with abdominal pain, tenderness and distension. Other less common signs and symptoms can present at first contact with the condition. However, due to its very poor prognosis, in most cases there is no return of jaundice with treatment. More than 90 percent of patients with cholangiocarcinoma present with advanced disease, which makes treatment difficult and aggressive. Only 20 percent of patients with cholangiocarcinoma present with a curable disease and only 10 percent survive 5 years.
Cholangiocarcinoma often presents with advanced disease and early recurrence. The data suggest that resection of CCA should be considered early to halt the local spread. This should improve survival.
This experience shows that melphalan/HDS can safely be completed in patients with advanced intrahepatic bile duct cancer who previously had undergone curative resective hepatectomy or hepatectomy with curative intent. However, in carefully selected patients, this option should be considered as one of the alternative options in the management of intrahepatic bile duct cancer. Findings from a recent study of the present study suggest that the administration of a melphalan-based combination chemotherapy with liver resection in selected patients with advanced intrahepatic bile duct cancer represents an effective treatment modality.
In the absence of convincing evidence to support a benefit for melphalan/HD regimen, the evidence for benefit of melphalan in the treatment of unresected cholangiocarcinoma is weak. Given the current costs and the limitations to further investigation of a novel therapy, the incremental cost (around 500 000) should be balanced against the benefit (about 200 per life year). Further work is required to define the most appropriate treatment for patients with unresectable cholangiocarcinoma.
The average age of diagnosis is 59. We conclude that the 5-year survival rate is approximately 70%. Survival rates depend on the stage of the disease when it is diagnosed (as well as the underlying cause) and is very poor once it has metastasized. Most surviving patients are able to continue with normal tasks like being self-employed, or work in positions that afford them basic amenities like food, health care, lodging, and transportation. Survival can be poor, however, and the prognosis depends on the extent of the disease at diagnosis.
A limited number of patients with cholangiocarcinoma have advanced tumor and are likely to benefit from therapies to treat metastatic disease. The most appropriate candidates should be selected according to the patient's baseline characteristics and the efficacy of the treatment, which would minimize the number of patients assigned to inappropriate trials.
We found that the majority of the trials evaluating hds-containing regimens used both hds (55%, n = 15) and the standard of cytotoxicity (27%, n = 7). No trial had utilized either of the two regimens solely.